Section I: Vascular rings
Definition
A vascular ring is a congenital anomaly in which the aortic arch and its branches completely or incompletely encircle and compress the trachea, esophagus, or both.
Historical note
Double aortic arch was apparently first described by Hommel in 1737 (cited by Turner) and a century later by Von Siebold. , Wolman is credited with describing the syndrome of tracheal and esophageal compression produced by a double arch in 1939. A description of a patient with dysphagia thought to be due to a retroesophageal right subclavian artery (RSA) was published in 1794 by Bayford, although the vessel was illustrated to pass between the esophagus and trachea rather than in its actual position posterior to the esophagus. ,
Modern interest in these anomalies was prompted by the first surgical correction of a double aortic arch by Robert Gross at Boston Children’s Hospital in 1945. Gross also coined the term “vascular ring,” was the first to divide the left ligamentum in a patient with a right aortic arch, and was the first to suspend a brachiocephalic artery that was compressing the trachea. The first successful diagnosis and repair of a pulmonary artery sling was reported by Willis Potts in 1954 at Children’s Memorial Hospital in Chicago. The basis for radiologic diagnosis was initially described by Edward Neuhauser, Chief of Radiology at Boston Children’s Hospital.
The complex development and regression of the aortic arches during fetal development were elucidated by Congdon in 1922, but until Gross’s pioneering surgical work, this information was little used by clinicians. In 1948, Jessee Edwards from the Mayo Clinic introduced the hypothetical double aortic arch scheme to conceptualize the numerous anomalies of the arch complex. This was further elaborated by Kirklin and Clagett in 1950 and by Stewart, Kincaid, and Edwards in 1964. In 1999, Momma and colleagues, followed by McElhinney and colleagues, identified chromosome 22q11 deletions associated with isolated anomalies of laterality or branching of the aortic arch. ,
Morphology
Variations in the arrangement of the ascending, transverse, and descending aorta and its branches are numerous in patients with vascular rings. Many of these may produce compression of the trachea, esophagus, or both, and are of surgical importance. They may be grouped as (1) complete or (2) incomplete vascular rings. Of 373 patients with a vascular ring or pulmonary artery sling that we reported from Chicago, 42% had a double aortic arch, 46% had a right aortic arch with left ligamentum, and 12% had a pulmonary artery sling. A summary of vascular rings associated with right aortic arch is seen in ( Fig. 39.1 ).
Vascular ring associated with right aortic arch. Center drawing depicts a double aortic arch with descending thoracic aorta as a midline or left-sided structure. Ligamentum arteriosum ( dashed line ) is shown originating from junction of right or left arch and descending aorta. Right-sided ligamentum arteriosum to right pulmonary artery usually forms in association with a right-sided descending aorta. Left-sided ligamentum arteriosum is the common configuration connecting midline or left descending thoracic aorta to left pulmonary artery near bifurcation. The four possible sites ( dotted lines ) of regression (interruption) of left arch during fetal development are shown. Various types of right aortic arch are depicted depending on site of interruption. Vascular ring results from a retroesophageal component of left arch giving rise to the ligamentum. A, Aortic arch branches arise in mirror image of normal. Anterior ligamentum arteriosum courses from brachiocephalic artery to proximal left pulmonary artery. There is no vascular ring. B, Left arch regresses just distal to left subclavian artery, leaving a retroesophageal aortic diverticulum. Ligamentum (ductus) arteriosum arises posteriorly from descending aorta and courses to left pulmonary artery, completing a vascular ring. C, Left arch regresses between left common carotid and left subclavian arteries, leaving a retroesophageal subclavian artery, with ligamentum (ductus) arteriosum forming a complete vascular ring. D, Left arch regresses between right arch and left common carotid artery. Complete vascular ring is present in this rare anomaly.
Complete vascular rings
Double aortic arch.
In patients with a double aortic arch, the ascending aorta arises normally, but as it leaves the pericardium it divides into two branches, a left and right aortic arch, that join posteriorly to form the descending aorta ( Fig. 39.2 ). The left aortic arch passes anteriorly and to the left of the trachea in the usual position and is joined by the ductus arteriosus (or more often a ligamentum arteriosum), where it becomes the descending aorta. The right aortic arch passes to the right and then posterior to the esophagus to join the descending aorta, thus completing the vascular ring. The descending aorta is most commonly on the right when there is a double aortic arch, but it can cross over to the left side. Alternatively, the descending aorta may be essentially a midline structure.
Double aortic arch with right dominant arch. Right common carotid and right subclavian arteries arise from the right aortic arch. Left common carotid and left subclavian arteries arise from smaller left aortic arch. The two arches join as the descending thoracic aorta, forming a complete vascular ring. In this example the right arch is dominant.
The right aortic arch gives origin to two vessels, the right common carotid and right subclavian arteries, and the left aortic arch gives origin to the left common carotid and left subclavian arteries in that order. The right aortic arch is usually (80% of cases ) larger than the left aortic arch, which often becomes narrow or atretic at its distal portion beyond the origin of the left subclavian artery). This part of the arch may remain patent or become a fibrous cord that joins the descending aorta, sometimes at the site of a Kommerell diverticulum (KD). A KD is an embryologic remnant of the left fourth arch that often becomes aneurysmal over time. The ligamentum in these cases passes from the adjacent proximal part of the main pulmonary artery to the arch itself. In approximately 10% of double aortic arch cases the left aortic arch is larger than the right aortic arch, which although smaller in its distal part after the origin of the RSA, is rarely atretic. The size of the right and left aortic arches is nearly equal (balanced) in about 5% of cases ( Fig. 39.3 ). Associated cardiovascular anomalies are uncommon.
CT scan of double aortic arch, balanced arches. This is from an 8-month-old who presented with noisy breathing and recent increased work of breathing. The right aortic arch was successfully divided via a right thoracotomy. The tracheal narrowing was caused by external compression. There was no intrinsic tracheal stenosis. LAA , left aortic arch; RAA , right aortic arch; Ao, aorta.
Right aortic arch with left ligamentum.
When there is a right aortic arch with a retroesophageal left subclavian artery, a vascular ring is usually present, but the anatomic details vary depending on site of regression (interruption) of the embryonic left aortic arch and the location of the left ligamentum (See Fig 39.1 ). When there is a right aortic arch with mirror image branching, the location of the ligamentum determines whether there is a vascular ring. The aortic arch branches arise in mirror image of normal. If the interruption of the embryonic left aortic arch occurs distal to the ductus arteriosus, the ligamentum courses from the anterior brachiocephalic artery to the proximal left pulmonary artery (LPA) and a vascular ring is not formed ( Fig. 39.4 ). This type is particularly common in tetralogy of Fallot and common arterial trunk.
Right aortic arch with mirror image branching. This anomaly is frequently associated with tetralogy of Fallot and truncus arteriosus. The ligamentum arteriosum is anterior, coursing from brachiocephalic artery to pulmonary artery. There is no vascular ring.
If the interruption of the left aortic arch is proximal (upstream) to the ligamentum (ductus arteriosus), the left-sided ligamentum extends from the upper descending thoracic aorta, around the esophagus, and forward to the pulmonary artery. A vascular ring is formed by the ascending portion of the right aortic arch and brachiocephalic artery anteriorly, by the aorta posteriorly, and by the ligamentum laterally. In the surgical series from Chicago, this anomaly represented one-third of right aortic arch vascular rings.
If the interruption of the left aortic arch occurs between the left subclavian and left common carotid arteries, there will be a retroesophageal left subclavian artery with a left ligamentum creating a vascular ring ( Fig. 39.5 ). The first branch of the right aortic arch is the left common carotid artery, and the descending aorta gives origin to the retroesophageal left subclavian artery as the fourth branch. The ductus or ligament inserts adjacent to the left subclavian artery. The descending aorta usually is on the right side. This is the most common type of vascular ring associated with a right aortic arch , accounting for about two-thirds of right aortic arch vascular rings. If the transverse aortic arch crosses over to a left-sided descending aorta, a circumflex aorta is created. In very rare cases, a left brachiocephalic artery originates from the descending aorta and there is a left ligamentum connecting from the descending aorta to the main pulmonary artery creating a vascular ring. The most frequently associated cardiac anomalies are ventricular septal defect and tetralogy of Fallot.
Right aortic arch with retroesophageal left subclavian artery and left ligamentum connecting the main pulmonary artery and descending thoracic aorta causing a complete vascular ring. R, right; RC, right carotid; RSA, right subclavian artery; LSA, left subclavian artery.
Kommerell Diverticulum: As noted earlier, a KD is an embryologic remnant of the distal left fourth arch. It was first described by a German radiologist named Burckhard Kommerell in 1936. He described a patient with a left aortic arch and an aberrant right subclavian artery from the distal aorta arising from a “diverticulum.” In vascular ring patients with a right aortic arch it has become clear with the advent of advanced imaging (computed tomography [CT], magnetic resonance imaging [MRI]) that a KD often serves as the origin of a retroesophageal left subclavian artery ( Fig. 39.6 ). A KD can independently cause symptoms by occupying space in the mediastinum and compressing adjacent structures such as the trachea and esophagus ( Fig. 39.7 ). Furthermore, the KD can enlarge over time and rarely rupture or cause an aortic dissection. If a KD is discovered on the preoperative imaging in a patient with a vascular ring, it is important to address it at the time of the surgical repair. Although most frequently found in patients with a right aortic arch, they can also be found in patients with a double aortic arch.
CT scan of a child with a right aortic arch (RAA), retroesophageal left subclavian artery (LSCA), and large Kommerell diverticulum (KD). This is a posterior view, and the trachea is shown in yellow. The small protrusion at the junction between the KD and LSCA is the insertion site of the ligamentum.
Axial CT image of a child with a right aortic arch (RAA), retroesophageal left subclavian artery, and left ligamentum. A large Kommerell diverticulum (KD) serves as the origin of the left subclavian artery. The KD is independently compressing the esophagus and trachea.
Circumflex Aorta: When there is a right aortic arch but the transverse aortic arch passes behind the trachea above the carina to a left-sided descending aorta a “circumflex aorta” is created ( Fig. 39.8 ). A circumflex aorta can also occur with a double aortic arch when the right aortic arch is dominant and in a similar fashion passes behind the trachea to a left-sided descending aorta ( Fig. 39.9 ). In these patients, simple division of the left ligamentum or left aortic arch may not provide relief of respiratory symptoms because of the posterior compression of the trachea by the aorta as it passes from right to left above the carina ( Fig. 39.10 ). For these patients, the operative procedure of choice is the aortic uncrossing . This clever operation was first described by Robotin and colleagues from Paris, France. Through a median sternotomy and with the use of cardiopulmonary bypass (CBP), the distal transverse arch is transected. The aorta is mobilized under the trachea and anastomosed to the left side of the ascending aorta in front of the trachea.
This computed tomography angiogram (CTA) demonstrates the posterior view of a 4.6-year-old child with a right cervical aortic arch, left ligamentum arteriosum, and retroesophageal left subclavian artery. The descending thoracic aorta crossing right to left posterior to the trachea and superior to the carina is clearly seen on this CTA. This posterior compression of the trachea is not relieved by ligamentum division.
(Reprinted with permission: Russell HM, Rastatter JC, Backer CL. The aortic uncrossing procedure for circumflex aorta. Oper Tech Thorac Cardiovasc Surg . 2013; 18:15-31). L, left; R, right; S, superior; I, inferior.
Double aortic arch with circumflex aorta. Preoperative CT scan in a child with noisy breathing, harsh cough, and multiple episodes of recurrent bronchitis. There is a right dominant double aortic arch, stenosis of the distal left arch and the left subclavian artery. Because the distal transverse aorta passes behind the trachea above the carina from right to left to join a left-sided descending aorta, a circumflex aorta is created.
Double aortic arch, right arch dominant, circumflex aorta. This axial image shows how the aorta compresses the posterior aspect of the membranous trachea. This posterior compression of the trachea by the aorta is one of the key diagnostic features that should lead the clinician to recommend an aortic uncrossing rather than simple ligamentum or left aortic arch division alone.
Left aortic arch and right descending aorta.
A vascular ring is formed in the uncommon combination of left aortic arch, right descending aorta, and right ligamentum. The left aortic arch crosses behind the esophagus. In combination with a right patent ductus arteriosus or right ligamentum arteriosum, a vascular ring is formed. , This is essentially a “mirror image” of a right aortic arch with a left ligamentum.
Cervical aortic arch.
Cervical aortic arch is a developmental entity consisting of persistence of the right or left third branchial arch and regression of the fourth branchial arch. The cervical aortic arch consists of a cervical position of the apex of the aortic arch with separate origin of the contralateral carotid artery, a retroesophageal descending aorta coursing contralaterally to the arch, and anomalous origin of the subclavian artery from the descending aorta. The cervical arch is most commonly right-sided. The aorta is usually redundant and crosses to the opposite side posterior to the esophagus. The retroesophageal segment of the aorta may be tortuous or severely narrowed. A vascular ring is formed when there is an aberrant subclavian artery on the side contralateral to the aortic arch and a ligamentum arteriosum. Aneurysm formation in the cervical arch has been reported. Abnormalities of brachiocephalic arterial branching and arch laterality are common in patients with cervical aortic arch. Rarely described is a left cervical aortic arch, right ligamentum arteriosum, and right descending aorta forming the vascular ring.
Incomplete vascular rings
Left aortic arch and retroesophageal right subclavian artery.
The relatively common (0.5% of the general population ) retroesophageal RSA arising as the fourth branch of an otherwise normal left aortic arch and passing upward and to the right behind the esophagus can be in rare cases a cause of dysphagia (dysphagia lusoria or “difficulty swallowing due to a trick of nature”) ( Fig. 39.11 ). , This condition does not form a complete ring but can still cause dysphagia because of compression of the esophagus by the subclavian artery as it traverses from left to right anterior to the spine and posterior to the esophagus. Because both this anatomy and dysphagia are so common, one must be very careful to attribute the dysphagia to an aberrant RSA prior to recommending an operation. The dysphagia, if indeed caused by the aberrant RSA, can be relieved by dividing and reimplanting the RSA.
Left aortic arch with aberrant distal origin of the right subclavian artery as the last branch off the arch of the aorta. The right subclavian artery passes posterior to the esophagus. RSA, right subclavian artery.
Tracheal compression by the brachiocephalic (innominate) artery.
In a child with a left aortic arch, the brachiocephalic (innominate) artery may be drawn taut across the anterior wall of the trachea, an uncommon cause of respiratory obstruction. , It is not known why the brachiocephalic artery occasionally compresses the trachea in this manner. Presumably, the brachiocephalic artery originates more posteriorly from the aortic arch than usual, so it compresses the trachea as it courses from a left and posterior location over the anterior trachea as it travels to the right side of the body.
Ductus arteriosus sling.
Binet and colleagues described an infant with respiratory obstruction in which an anomalous vessel (presumed to be the ductus arteriosus) originated from the right pulmonary artery (RPA), crossed to the left between the esophagus and trachea, and joined the descending aorta adjacent to the origin of a retroesophageal RSA.
Severe malrotation of heart with patent ductus arteriosus.
Compression of the lower trachea can occur with a normal left arch when there is severe malrotation of the heart into the right chest in association with agenesis or hypoplasia of the right lung. , Scherer and Westcott described a patient with dextrocardia and normal lungs in whom the pulmonary trunk lay anterior to the trachea and somewhat to the right. The patent ductus arteriosus connecting with a normally positioned descending aorta pulled the pulmonary trunk backward, compressing the front of the trachea. Compression was relieved by dividing the patent ductus arteriosus.
Clinical features and diagnostic criteria
Symptoms and signs
Symptoms of a vascular ring relate to the consequences of tracheal and esophageal compression. Presentation is usually within the first 6 months of life and often within the first month of life. Inspiratory stridor may be present at birth, often in association with an expiratory wheeze and tachypnea. Stridor may be worse in various positions—for example, when the baby is lying on his or her back rather than side. Often, stridor is relieved by extending the neck. The baby’s cry may be hoarse and, in the absence of frank stridor, the breathing noisy. A persistent barking cough is frequently present. There may be episodes of apnea, cyanosis, and unconsciousness, referred to as an ALTE (apparent life-threatening event). When obstruction is severe, subcostal retraction is obvious. Recurrent respiratory infections are common and aggravate the respiratory obstruction. When obstruction is less severe, obstructive symptoms may be apparent only when the child has an upper respiratory infection. Infants do not usually have trouble swallowing liquid formula or breast milk but develop dysphagia for solids once they are offered.
In general terms infants with a double aortic arch present with symptoms earlier than infants with a right aortic arch and a left ligamentum. Most infants with a double aortic arch will present with symptoms in the first month of life. Patients with a right aortic arch tend to present with symptoms slightly later at 1 to 6 months of age. Brachiocephalic artery compression syndrome also presents usually in the first months of life. Typical symptoms of a vascular ring include: stridor (60%), recurrent upper respiratory infections (30%), cough (10%), dysphagia (15%), and respiratory distress (10%). Brachiocephalic artery compression syndrome presents with stridor, recurrent upper respiratory infections, and apneic episodes. Other frequent symptoms are wheezing and dyspnea on exertion in older children. Dysphagia typically does not occur until the child is old enough to take solid foods. There are many anecdotal stories of patients who have learned to chew their food very slowly and are always the last one to finish eating because of the esophageal compression from the vascular ring. A careful history often reveals episodes of “croup” and “asthma” being treated with inhalers.
Dysphagia is sometimes attributed to retroesophageal origin of the RSA from the upper descending thoracic aorta. The artery courses to the right, posterior to the esophagus, producing an indentation of the esophagus that can possibly cause dysphagia, but that is usually not the cause. Should the subclavian artery become ectatic or aneurysmal later in life, difficulty in swallowing can occur. Given how common this anomaly is, it is only in rare cases that the RSA is the actual cause of dysphagia.
Symptomatic vascular rings manifesting in adults are rare, and reports usually emphasize dysphagia as the predominant symptom. Grathwohl reviewed case reports of 24 adults with vascular rings. Saran and colleagues from the Mayo Clinic reviewed 65 adult patients with vascular rings. Dysphagia was the most common symptom.
Chest radiograph
The first diagnostic study in a child with a suspected vascular ring is usually an anteroposterior and lateral chest x-ray. The mediastinum should be examined for signs of a right or left aortic arch. This is assessed by the location of the apex of the aortic arch in relationship to the trachea. When the location of the aortic arch is indeterminate a double aortic arch should be suspected. Unilateral hyperinflation of the right lung may indicate a pulmonary artery sling compressing the right main bronchus.
Esophagogram
For many years barium esophagogram was the diagnostic procedure of choice for vascular rings. That completely changed with the introduction of CT and MRI. We now recommend barium esophagograms only in unusual cases where the indications for operative intervention are borderline. Esophagograms are also useful for patients with a left aortic arch and aberrant RSA. The use of a barium tablet as part of the esophagram study can demonstrate that the compression from the aberrant subclavian artery is creating a significant obstruction that will benefit from surgical intervention ( Fig. 39.12 ). With double aortic arch, the esophagram shows left- and right-sided indentations, with the indentation for the right arch usually higher and deeper. In contrast, a retroesophageal left subclavian artery arising from a right aortic arch produces a narrower esophageal impression that courses upward and leftward. Right aortic arch and left ligamentum arteriosum show more marked right-sided than left-sided indentation. Although historically (before 1990) surgeons would operate with just a chest x-ray and a barium swallow, we now recommend preoperative CT or MRI on all patients prior to the operation.
In this patient with a left aortic arch and an aberrant right subclavian artery a barium tablet can be seen unable to pass in the esophagus because of external compression from the right subclavian artery.
Bronchoscopy
Bronchoscopy is quite important and it will identify the degree of tracheomalacia and show the sites of pulsatile external compression ( Fig. 39.13 ) We recommend either preoperative or intraoperative bronchoscopy on all vascular ring patients, particularly those with a pulmonary artery sling. The findings can help predict how severe the tracheomalacia is and the anticipated postoperative recovery. Rarely, additional significant pathology such as a foreign body, complete tracheal ring, or anomalous bronchial origin is discovered. For patients with a pulmonary artery sling, complete cartilage tracheal rings are very common.
Bronchoscopic image of a double aortic arch with anterior compression on both sides of the trachea.
Two-dimensional echocardiogram
Two-dimensional echocardiogram can be helpful in the preliminary diagnosis of a vascular ring, at least in neonates and infants. However, echocardiogram alone is not enough for complete, precise anatomic diagnosis, which is why these patients need a CT or MRI. Echocardiogram is critically important for identifying associated cardiac anomalies. We recommend preoperative echocardiogram in all vascular ring patients to rule out cardiac anomalies.
Fetal echocardiogram
In the past decade fetal echocardiography has become quite facile in detecting vascular rings prior to the child being born. In particular, the three-vessel tracheal view has become a standard part of the level 2 obstetric scans. This allows for the preemptive monitoring of these patients and formal strategies for postnatal follow-up. The algorithm we developed in Chicago for the management of fetal diagnosis of a vascular ring is shown in Fig. 39.14 .
Algorithm for the management of patients diagnosed with a fetal vascular ring. DAA, double aortic arch; PA, pulmonary artery; RAA, right aortic arch; CT, computerized tomography.
Computed tomography
CT imaging is our preferred method of preoperative evaluation. One of the major advantages of CT imaging is that it can be performed very quickly (in a few seconds) and there is rarely, if ever, a need to sedate or intubate the child. Our strategy for infants is to “feed and swaddle” which works extremely well. A double aortic arch can be diagnosed if both limbs are patent and therefore contrast enhanced. If one of the limbs is atretic a careful interpretation of the arch vessels recognizing the typical arterial branching pattern can make the diagnosis. CT imaging will also reveal the anatomic details of a right aortic arch, including a KD when present and the anatomy of the transverse aorta, defining a circumflex aorta. It is also very good at imaging pulmonary artery sling, tracheal stenosis, and brachiocephalic artery compression.
Magnetic resonance imaging
The limitation of MRI is the length of time it currently takes to perform the study. This may require that the child be sedated and intubated. In addition, MRI does not provide as clear an image of the trachea as CT does. The main advantage of MRI is to avoid radiation, but the newer CT protocols have reduced the radiation exposure to levels that are quite low and are very safe.
Natural history
Vascular rings account for 1.5% of surgical cases of congenital heart disease. With fetal ultrasound and frequently used advanced imaging modalities (CT, MRI) vascular rings are now known to be more common than what was previously thought. Evans and colleagues performed a comprehensive review of fetal echocardiograms in Southern Nevada. They found that the prevalence figure for vascular rings is 7 per 10,000 live births, very similar to the prevalence figure for tetralogy of Fallot. The nearly 90% fetal detection rate allows for potentially altering the natural history of a congenital cardiac lesion that was previously diagnosed only after the onset of symptoms.
Although there are some reports of children eventually having improvement in their symptoms as they grow, this has not been my experience. In fact, I have seen many children where the diagnosis has been delayed resulting in multiple hospitalizations for pneumonia and recurrent upper respiratory infections. I recommend surgical intervention in all symptomatic patients with a vascular ring. The main exception to this is the rare asymptomatic patient with a right aortic arch and left ligamentum who has minimal tracheal compression by CT and bronchoscopy, and minimal evidence of esophageal compression by barium swallow.
Technique of operation
Double aortic arch
In cases of double aortic arch with a dominant right aortic arch and left-sided ligamentum, the operation is performed through a left thoracotomy via the third or fourth interspace. A right thoracotomy approach is used when the left arch is dominant. Median sternotomy may be used when coexisting cardiac anomalies require repair.
When the right aortic arch is dominant, the left aortic arch and ligamentum are dissected via a left thoracotomy. The ligamentum is doubly ligated and divided, taking care to avoid injury to the recurrent laryngeal nerve. The left arch is then divided between clamps close to its junction with the descending aorta ( Fig. 39.15 ). The stumps are oversewn with two rows of 5-0 or 6-0 polypropylene sutures ( Fig. 39.16 ). It is important to use staged division of a patent aortic arch to prevent the divided stump from slipping through the clamp. In this and other operations for relief of vascular rings, “all strands or bands of tissue which form a part of the constricting mechanism” must be dissected away from the trachea or esophagus. It is also important to leave the pleura open at the end of the case to prevent scarring and recurrence of compression. The operation is completed by closing the chest wound in layers after inserting a single soft Blake drain. The patient should be extubated in the operating room if possible.
Surgical approach to double aortic arch, right arch dominant. The ligamentum has been divided and oversewn, and vascular clamps are on the patent distal left aortic arch. The proximal clamp is a Potts ductus clamp and the distal clamp is a Castaneda clamp. The first suture is a horizontal mattress suture. The second suture is a running “baseball” suture. Note the majority of the suture line has been completed prior to transecting the patent arch (staged technique).
Completed procedure with distal arch and ligamentum divided.
Techniques have been developed to divide vascular rings using video-assisted thoracoscopy (VATS) and were first reported in 1995. Herrin and colleagues from Boston Children’s Hospital recently updated that preliminary experience. The VATS procedure based on that series seems to be almost exclusively indicated in patients with an atretic arch. Only 4 of their 115 patients had a patent aortic arch. Two factors have led to the reasons VATS has not caught on for division of vascular rings. The first is the anecdotal reports of catastrophic hemorrhage during attempted VATS procedures. The second is advanced imaging leading to an understanding that more than just simple atretic arch or ligamentum division is needed in many patients.
If the left aortic arch is dominant the approach is through a right thoracotomy in the 4th intercostal space. The right (smaller) arch is dissected out and divided between vascular clamps just as is illustrated for division of a left arch. The ligamentum does not need to be divided in these patients. This is fortunate as it is not easily accessible from the right side of the chest.
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